Vertebral muscular atrophy (SMA) is a neurodegenerative disease produced by low

Vertebral muscular atrophy (SMA) is a neurodegenerative disease produced by low levels of Survival Motor Neuron (SMN) protein that affects alpha motoneurons in the spinal cord. In these motoneurons an increased BYL719 Notch signaling was found, as denoted by increased NICD levels and reduced expression of the proneural gene neurogenin 3, whose transcription is negatively regulated by Notch. Together, these findings may be relevant to understand some pathologic attributes of SMA motoneurons. model of SMA, the SMN7 mouse model, we also studied the expression of Notch ligands in reactive astrocytes in relation with the potential activation of the Notch signaling